-
-
关键词:
- 血栓性血小板减少性紫癜 /
- ADAMTS13 /
- 血浆置换
Abstract: Thrombotic thrombocytopenic purpura (TTP) is a relatively rare clinical disease.It belongs to thrombotic microangiopathy (TMA) as well as uremic hemolytic anemia (HUS) , but its clinical manifestations are not the same.This article elaborates the latest advances in clinical manifestations, laboratory tests, diagnosis and differential diagnosis, and treatment of TTP.-
Key words:
- TTP /
- ADAMTS13 /
- plasmapheresis
-
-
[1] Moschcowitz E.Hyaline thrombosis of the terminal arterioles and capillaries:a hitherto undescribed disease[J].Proc N Pathol Soc, 1924, 24 (1):21.
[2] Furlan M, Robles R, Galbusera M, et al.von Willebrand factor-cleaving protease in thrombotic thrombocytopenic purpura and the hemolytic-uremic syndrome[J].N Engl J Med, 1998, 339 (22):1578-1584.
[3] Tsai H M, Lian E C.Antibodies to von Willebrand factor-cleaving protease in acute thrombotic thrombocytopenic purpura[J].N Engl J Med, 1998, 339 (22):1585-1594.
[4] Scully M, Yarranton H, Liesner R, et al.Regional UKTTP registry:correlation with laboratory ADAMTS13 analysis and clinical features[J].Br J Haematol, 2008, 142 (5):819-826.
[5] Lotta L A, Mariani M, Consonni D, et al.Different clinical severity of first episodes and recurrences of thrombotic thrombocytopenic purpura[J].Br JHaematol, 2010, 151 (5):488-494.
[6] Fujimura Y, Matsumoto M.Registry of 919patients with thrombotic microangiopathies across Japan:database of Nara Medical University during 1998-2008[J].Intern Med, 2010, 49 (1):7-15.
[7] Kremer Hovinga J A, Vesely S K, Terrell D R, et al.Survival and relapse in patients with thrombotic thrombocytopenic purpura[J].Blood, 2010, 115 (8):1500-1511.
[8] Jang M J, Chong S Y, Kim I H, et al.Clinical features of severe acquired ADAMTS13 deficiency in thrombotic thrombocytopenic purpura:the Korean TTPregistry experience[J].Int J Hematol, 2011, 93 (2):163-169.
[9] Deford C C, Reese J A, Schwartz L H, et al.Multiple major morbidities and increased mortality during long-term follow-up after recovery from thrombotic thrombocytopenic purpura[J].Blood, 2013, 122 (12):2023-2029.
[10] Blombery P, Kivivali L, Pepperell D, et al.TTP registry steering committee.Diagnosis and management of thrombotic thrombocytopenic purpura (TTP) in Australia:findings from the first 5years of the Australian TTP/thrombotic microangiopathy registry[J].Intern Med J, 2016, 46 (1):71-79.
[11] Mariotte E, Azoulay E, Galicier L, et al.French Reference Center for Thrombotic Microangiopathies.Epidemiology and pathophysiology of adulthood-onset thrombotic microangiopathy with severe ADAMTS13 deficiency (thrombotic thrombocytopenic purpura):a cross-sectional analysis of the French national registry for thrombotic microangiopathy[J].Lancet Haematol, 2016, 3 (5):e237-e245.
[12] Levy G G, Nichols W C, Lian E C, et al.Mutations in a member of the ADAMTS gene family cause thrombotic thrombocytopenic purpura[J].Nature, 2001, 413 (6855):488-494.
[13] Berangere S J, Paul C.Agn es Veyradier, Thrombotic thrombocytopenic purpura[J].Blood, 2017, 129 (21):2836-2846.
[14] Sadler J E.What's new in the diagnosis and pathophysiology of thrombotic thrombocytopenic purpura[J].Hematol Am Soc Hematol Educ Program, 2015, 2015:631-636.
[15] Matsumoto M, Yagi H, Ishizashi H, et al.The Japanese experience with thrombotic thrombocytopenic purpura-hemolytic uremic syndrome[J].Semin Hematol, 2004, 41 (1):68-74.
[16] Studt J D, Kremer Hovinga J A, Antoine G, et al.Fatal congenital thrombotic thrombocytopenic purpura with apparent ADAMTS13 inhibitor:in vitro inhibition of ADAMTS13 activity by hemoglobin[J].Blood, 2005, 105 (2):542-544.
[17] Ono T, Mimuro J, Madoiwa S, et al.Severe secondary deficiency of von Willebrand factorcleaving protease (ADAMTS13) in patients with sepsis-induced disseminated intravascular coagulation:its correlation with development of renal failure[J].Blood, 2006, 107 (2):528-534.
[18] Peigne V, Azoulay E, Coquet I, et al.The prognostic value of ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1repeats, member13) deficiency in septic shock patients involves interleukin-6and is not dependent on disseminated intravascular coagulation[J].Crit Care, 2013, 17 (6):R273.
[19] Lotta L A, Garagiola I, Palla R, et al.ADAMTS13 mutations and polymorphisms in congenital thrombotic thrombocytopenicpurpura[J].Hum Mutat, 2010, 31 (1):11-19.
[20] Hanby H A, Zheng X L.Current status in diagnosis and treatment of hereditary thrombotic thrombocytopenic purpura[J].Hereditary Genet, 2014, 3 (1):e108.
[21] Martino S, Jamme M, Deligny C, et al.French Reference Center for Thrombotic Microangiopathies.Thrombotic thrombocytopenic purpura in black people:impact of ethnicity on survival and genetic risk factors[J].PLoS One, 2016, 11 (7):e0156679.
[22] Benhamou Y, Boelle P Y, Baudin B, et al.Reference Center for Thrombotic Microangiopathies.Experience of the French Thrombotic Microangiopathies ReferenceCenter.Cardiac troponin-I on diagnosis predicts early death and refractoriness in acquired thrombotic thrombocytopenic purpura[J].J Thromb Haemost, 2015, 13 (2):293-302.
[23] Zafrani L, Mariotte E, Darmon M, et al.Acute renal failure is prevalent in patients with thrombotic thrombocytopenic purpura associated with low plasma AD-AMTS13 activity[J].J Thromb Haemost, 2015, 13 (3):380-389.
[24] George J N.Measuring ADAMTS13 activity in patients with suspected thrombotic thrombocytopenic purpura:when, how, and why?[J].Transfusion, 2015, 55:11.
[25] Jamme M, Rondeau E.The PLASMIC score for thrombotic thrombocytopenic purpura[J].Lancet Haematol, 2017, 4 (4):e148.
[26] Risam S.Management of hemolytic-uremic syndrome in children[J].Int J Nephrol Renovasc Dis, 2014, 7:231-239.
[27] Scully M, Hunt B J, Benjamin S, et al.British Committee for Standards in Haematology.Guidelines on the diagnosis and management of thrombotic thrombocytopenic purpura and other thrombotic microangiopathies[J].Br J Haematol, 2012, 158 (3):323-335.
[28] Froissart A, Buffet M, Veyradier A, et al.French Thrombotic Microangiopathies Reference Center.Experience of the French Thrombotic Microangiopathies Reference Center.Efficacy and safety of first-line rituximab in severe, acquired thrombotic thrombocytopenic purpura with a suboptimal response to plasma exchange[J].Crit Care Med, 2012, 40 (1):104-111.
[29] Bell W R, Braine H G, Ness P M, et al.Improved survival in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome.Clinical experience in 108patients[J].N Engl J Med, 1991, 325 (6):398-403.
[30] Masanori M, Yoshihiro F, Hideo W, et al.Diagnostic and treatment guidelines for thrombotic thrombocytopenic purpura (TTP) 2017in Japan[J].Int J Hematol, 2017, 106 (1):3-15.
[31] Balduini C L, Gugliotta L, Luppi M, et al.Italian TTPStudy Group.High versus standard dose methylprednisolone in the acute phase of idiopathic thrombotic thrombocytopenic purpura:a randomized study[J].Ann Hematol, 2010, 89 (6):591-596.
[32] Elliott M A, Heit J A, Pruthi R K, et al.Rituximab for refractory and or relapsing thrombotic thrombocytopenic purpura related to immune-mediated severe AD-AMTS13-deficiency:a report of four cases and a systematic review of the literature[J].Eur J Haematol, 2009, 83 (4):365-372.
[33] Scully M, McDonald V, Cavenagh J, et al.A phase 2study of the safety and efficacy of rituximab with plasma exchange in acute acquired thrombotic thrombocytopenic purpura[J].Blood, 2011, 118 (7):1746-1753.
[34] Jasti S, Coyle T, Gentile T, et al.Rituximab as an adjunct to plasma exchange in TTP:a report of 12cases and review of literature[J].J Clin Apher, 2008, 23 (5):151-156.
[35] Ling H T, Field J J, Blinder M A.Sustained response with rituximab in patients with thrombotic thrombocytopenic purpura:a report of 13cases and review of the literature[J].Am J Hematol, 2009, 84 (7):418-421.
[36] de la Rubia J, Moscard'o F, G'omez M J, et al.Grupo Espa-nol de Af'eresis.Efficacy and safety of rituximab in adult patients with idiopathic relapsing or refractory thrombotic thrombocytopenic purpura:results of a Spanish multicenter study[J].Transfus A-pheresis Sci, 2010, 43 (3):299-303.
[37] Page E E, Kremer Hovinga J A, Terrell D R, et al.Rituximab reduces risk for relapse in patients with thrombotic thrombocytopenic purpura[J].Blood, 2016, 127 (24):3092-3094.
[38] Scully M, Cohen H, Cavenagh J, et al.Remission in acute refractory and relapsing thrombotic thrombocytopenic purpura following rituximab is associated with a reduction in IgG antibodies to ADAMTS-13[J].Br JHaematol, 2007, 136 (3):451-461.
[39] Cuker A.Adjuvant rituximab to prevent TTP relapse[J].Blood, 2016, 127 (24):2952-2953.
[40] Chen J, Reheman A, Gushiken F C, et al.Nacetylcysteine reduces the size and activity of von Willebrand factor in human plasma and mice[J].J Clin Invest, 2011, 121 (2):593-603.
[41] Shortt J, Oh D H, Opat S S.ADAMTS13 antibody depletion by bortezomib in thrombotic thrombocytopenic purpura[J].N Engl J Med, 2013, 368 (1):90-92.
[42] Hie M, Gay J, Galicier L, et al.French Thrombotic Microangiopathies Reference Centre.Preemptive rituximab infusions after remission efficiently prevent relapses in acquired thrombotic thrombocytopenic purpura[J].Blood, 2014, 124 (2):204-210.
[43] Kappers-Klunne M C, Wijermans P, Fijnheer R, et al.Splenectomy for the treatment of thrombotic thrombocytopenic purpura[J].Br J Haematol, 2005, 130 (5):768-776.
[44] Hughes C, McEwan J R, Longair I, et al.Cardiac involvement in acute thrombotic thrombocytopenic purpura:association with troponin T and IgG antibodies to ADAMTS 13[J].J Thromb Haemost, 2009, 7 (4):529-536.
[45] Benhamou Y, Assi'e C, Boelle P Y, et al.Thrombotic Microangiopathies Reference Center.Development and validation of a predictive model for death in acquired severe ADAMTS13 deficiency-associated idiopathic thrombotic thrombocytopenic purpura:the French TMA Reference Center experience[J].Haematologica, 2012, 97 (8):1181-1186.
[46] George J N.How I treat patients with thrombotic thrombocytopenic purpura:2010[J].Blood, 2010, 116 (20):4060-4069.
-
计量
- 文章访问数: 68
- PDF下载数: 46
- 施引文献: 0
下载: